[CIS PIDD] [cis-pidd] hemophagocytic lymphohistiocytosis after HSCT

[Eleonora Gambineri]

Dear all, 

I would like to ask your advice for a patient affected by Thalassemia
who underwent haploidentical HSCT (PT-Cyclophosphamide) about one year
ago. She didn't suffer from classical GvHD, but she experienced
cytopenias (leucopenia and neutropenia, thrombocytopenia, anemia) that
we initially thought to be immune mediated, therefore she was treated
with steroids and Rituximab with partial response. At a recent BM
aspirate many histiocytes were found in the bone marrow. She is
presumably suffering from hemophagocytic lymphohistiocytosis after
transplantation (she also have high ferritin and triglicerid levels as
well as CRP). She was treated with Desametasone (10mg/mq) with partial
response (on red cells and platelets, but neutropenia persisted) and she
relapsed when the steroid was tapered. She is at the moment on CsA. 

Does anyone has experience on that? Any advice on further treatment?
Vincristine? VP-16? Re-transplant? Prognosis? 

Thanks a lot for your help! 

Best wishes,
Eleonora 

*******************************************************************
 Eleonora Gambineri, MD
 Researcher/Assistant Professor

 Department of "NEUROFARBA": Section of Child's Health
 University of Florence 

 Department of Haematology-Oncology: BMT Unit
 Department of Fetal and Neonatal Medicine: Rare Diseases,
 "Anna Meyer" Children's Hospital

 Viale Gaetano Pieraccini,24
 50139 FIRENZE
 ITALY
 Tel +39 055 5662405 (office)/055 5662738(BMT ward)
 Fax +39 055 4221012
 e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it
 ******************************************************************** 

 
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